Leap Motion Controller–based training for upper extremity rehabilitation in children and adolescents with physical disabilities: A randomized controlled trial

Study DesignRandomized controlled trial.IntroductionJuvenile idiopathic arthritis (JIA), cerebral palsy (CP), and brachial plexus birth injury (BPBI) are the most common disorders that cause upper extremity impairments in children and adolescents. Leap Motion Controller–based training (LMCBT) is a novel therapeutic method for upper extremity rehabilitation.Purpose of the StudyThe aim of the present study was to investigate the potential efficacy of an 8-week LMCBT program set as an upper extremity rehabilitation program by comparing conventional rehabilitation program in children and adolescents with physical disabilities such as JIA, CP, and BPBI.MethodsA randomized control trial which included children and adolescents of different disabilities (JIA, CP, BPBI) were grouped according to their diagnosis. All patients were randomized into 2 groups namely LMCBT (group I) and conventional treatment (group II) for the treatment (3 days/8 weeks). Duruoz Hand Index and Jebson Taylor Hand Function Test were used as primary outcomes. Secondary outcomes included the nine-hole peg test, Childhood Health Assessment Questionnaire, and assessments of grip and pinch strength using a dynamometer.ResultsOne hundred three patients were included in the study, and 92 of them completed the treatment. After treatment, significant differences were found in Childhood Health Assessment Questionnaire, Duruoz Hand Index, Jebson Taylor Hand Function Test, nine-hole peg test, and grip and pinch strength scores in almost all groups (effect size [ES] = 0.10 to −0.77 for group I and 0.09 to −0.70 for group II in CP; ES = 0.31 to 2.65 for the group I and 0.12 to 1.66 for group II in JIA; and ES = 0 to −0.44 for group I and 0.08 to −0.62 for group II in BPBI) (P < .05). Comparisons between LMCBT and conventional treatment groups showed similar results in all parameters in all disease groups (P > .05).ConclusionsThis study has quantitatively shown that LMCBT should be used as an effective alternative treatment option in children and adolescents with physical disabilities.     

RCT of a promising vocational/employment program for high-risk juvenile offenders

Juvenile offenders with substance use problems are at high risk for deleterious long-term outcomes. This study evaluated the capacity of a promising vocational and employment training program in the building sector (i.e., Community Restitution Apprenticeship-Focused Training, CRAFT) to mitigate such outcomes through enhanced employment and education. Participants were 97 high-risk juvenile offenders (mean age = 15.8 years) randomized to CRAFT versus education as usual (EAU) intervention conditions. Multi-method procedures measured employment, education, substance use, mental health, and criminal outcomes through a 30-month post-baseline follow-up. CRAFT was significantly more effective than EAU at increasing rates of youth employment and GED attendance. Intervention effects were not observed, however, for months employed, hours worked, or hourly wage. Measures of youth substance use, mental health symptoms, and criminal activity showed no favorable or iatrogenic effects. The potential of CRAFT was modestly supported, and suggestions were made for future research.     

Reference values of ECG parameters derived from 906 echocardiographically confirmed healthy Indian children: A population-based study from Gujarat

Introduction

There are few published studies on reference ranges of ECG parameters in children; some ethnic differences have been described.

Interleukin-1, -6, and -8 levels in juvenile chronic arthritis

Summary The immunoinflammatory pathogenesis of juvenile chronic arthritis (JCA) involves the activation of many pathways including various cytokines. We have evaluated the levels of interleukin (IL)-1, IL-6 and IL-8 in 29 JCA patients. The age range was 1–16 with a mean of 10.1. A disease activity score was developed on the basis of: 1.constitutional symptoms and/or morning stiffness, 2.presence of joint swelling, 3.warmth, 4.limited range of motion, and 5.joint pain. This score correlated very significantly with laboratory disease activity markers such as erythrocyte sedimentation rate (ESR) and CRP (both p=0.006) and also correlated with IL-1 and IL-6 levels. The levels of IL-1 decreased in four of the five patients with improved disease activity. IL-6 but not IL-1 correlated significantly with the number of inflamed joints (p=0.013); IL-6 also strongly correlated with rheumatoid factor supporting this cytokine's role in B cell induction (p=0). Haemoglobin values correlated negatively with the activity index, ESR, CRP, IL-1 and IL-6. IL-8 did not correlate with disease activity markers. In the systemic patients all cytokines tended to be higher. Our data suggest that interleukins 1 and 6 are effective in the pathogenesis of JCA. Whether cytokines may be used for monitoring therapy may be clarified with further studies.     

A case of planned pregnancy with an interruption in infliximab administration in a 27-year-old female patient with rheumatoid-factor-positive polyarthritis juvenile idiopathic arthritis which improved after restarting infliximab and methotrexate

We report a 27-year-old case of juvenile idiopathic arthritis (JIA) having been stopped infliximab during pregnancy. She was safely treated by infliximab therapy with premedications for preventing infusin reactions after her delivery, and then improved in the same manner as when she had been treated with infliximab therapy before pregnancy. As a result, it remains unclear whether or not we can use infliximab to control disease activities during pregnancy. In addition, it is also important to clarify whether or not premedications should be used when resuming infliximab treatment in such patients after pregnancy. These problems still remain controversial. More definitive data are needed in order to allow rheumatologists to better select the optimal TNF-alpha inhibitor therapy when treating pregnant JIA patients.     

Factors associated with lack of response to valproic acid monotherapy in juvenile myoclonic epilepsy

PurposeTo determine factors associated with lack of response to valproic acid (VPA) in juvenile myoclonic epilepsy (JME).MethodRetrospective analysis of clinical and EEG data of 201 patients with JME who had at least 3 years follow up was performed. Psychiatric evaluation was performed using ICD-10 by structured clinical interview. Patients were divided into two groups: VPA responders (seizure free for 2 or more years) and those with lack of response to VPA. Effect size for non-response and correlations for variables significantly different between the groups was performed, the findings were confirmed by ROC curves.ResultsThe mean duration of follow up was 7.75 (range 3–12) years; 55.2% were males. Focal semiologic features were noted is 16%. EEG was abnormal in 67%; focal EEG abnormalities were noted in 32.8%. Coexisting psychiatric disorders (PDs) were found in 33.3%. Lack of response to VPA was noted in 19%. Diagnosis of PDs and focal EEG abnormalities significantly increased the risk of VPA non-responsiveness by 5.54 (95% CI of 2.60–11.80; p < 0.0001) and 3.01 times respectively (95% CI of 1.40–6.47; p < 0.008). Diagnosis of PDs showed significant correlation (r = 0.332; p < 0.0001) and association (AUC 0.700; p < 0.0001) with lack of response to VPA. Though focal EEG abnormalities increased the chances, it did not correlate with lack of response to VPA.ConclusionLack of response to VPA was noted 19% of patients with JME. Coexisting PDs showed significant correlation and association with lack of response to VPA.     

儿童类风湿关节炎高贡献率易感基因的研究

目的通过对小儿类风湿关节炎全基因组基因拷贝数变异(copy number variations,CNVs)的研究,为该病易感基因筛选和发病机制研究提供理论基础。方法选取小儿类风湿关节炎患者(Juvenile rheumatoid arthritis,JRA)20例作为患病组,随机从体检中心选取无类风湿关节炎患病史健康儿童20例作为健康对照组。采用测序仪分别对类风湿关节炎患病组和健康组的DNA-pool进行全基因组重测序,用生物信息学软件对测序数据进行分析。结果我们采用CNVnator软件对患病组和健康组的测序数据进行检测,发现CNV位点分别为7479个和5201个。通过生物信息学分析筛选,患病组与健康组比较发现有825个CNVs位点,其中有192个位于内含子区域,有93个位于外显子区域,19个位于上游位置,25个位于下游位置。结论本研究揭示了儿童期类风湿关节炎患者的基因组拷贝数变异的变化,并发现了编码区的KIR3DL1、CD247、PPIP5K1、MIOX、ANK3、HK3基因可能为类风湿关节炎的易感基因。     

A hyper-ferritinemia syndrome evolving in recurrent macrophage activation syndrome,as an onset of amyopathic juvenile dermatomyositis: A challenging clinical case in light of the current diagnostic criteria

Juvenile dermatomyositis is an immune-mediated inflammatory multi-system disease involving mainly striated muscles and skin. Typical dermatological features are fundamental to establish the diagnosis, especially whenever the myopathy is very mild or absent, as it occurs in the form called as amyopathic juvenile dermatomyositis. Sometimes, systemic rheumatic diseases can develop a hyperferritinemia syndrome characterized by hemophagocytosis, namely macrophage activation syndrome, which represents a severe and life-threatening complication. Here, we describe a complex clinical history characterized by a hyper-ferritinemia syndrome after infectious mononucleosis, leading to recurrent episodes of macrophage activation syndrome. Finally, the late onset of several skin changes brought to a diagnosis of amyopathic juvenile dermatomyositis.